RESULTS
Twenty-one patients were diagnosed with CLE at our center between 2013 and 2020. One patient did not attend the follow-up meetings; therefore, 20 patients were ultimately included in the study. The median age at diagnosis was 3.2 years (range, 1 day–17 years), and the median age at admission to our clinic was 4.53 years (range, 21 days–17 years). Nine of the patients (45%) were female, and 11 (55%) were male. Seven (35%) patients had no symptoms at admission to our clinic. Fourteen patients (87.5%) presented with cough, and peroral antibiotics were administered with the diagnosis of pneumonia; before admission to our clinic.
Two patients (10%) were diagnosed with CXR, and thorax ultrasonography was performed during the postnatal period, with cystic lesions observed in the lungs in the fetal ultrasound. Three patients (15%) were diagnosed with suspected lung infection and moaning during the neonatal period, four patients (20%) were diagnosed with foreign body aspiration, and nine patients (45%) were diagnosed with recurrent and/or intractable lung infection.
One patient (5%) had a normal CXR and was diagnosed after a trauma. One patient was diagnosed with incidental CLE by CT performed because of cavities in the lung after pneumonia. With the exception of these two patients, all patients presented with localized increased aeration on the CXR. In the CXR and CT, two patients (10%) presented with mediastinal shift and secondary atelectasis, and three patients (15%) presented with mild mediastinal/tracheal deviation. All but three patients (15%) underwent thoracic CT before FB. On CT, left upper lobe 10 (50%), right middle lobe 3 (15%), right upper lobe 2 (10%), left lingula 1 (5%), left lower lobe 1 (5%) which may be compatible with CLE. There were two patients (%10) with multiple lesions which may be compatible with CLE. One 1 (5%) patient with right emphysematous areas which were observed in the upper and right middle lobes , and 1(5%) patient with bilateral emphysematous areas in the left lower and right upper lobes. CT demonstrated vascular compression in one (5%) patient (Table 1). None of the patients underwent ventilation-perfusion scintigraphy.
All the patients underwent FB to rule out primary and secondary causes that could lead to increased aeration. Macroscopically, FB revealed malacia in eight patients (40%), mucosal folds in seven patients (35%), and fish-mouth deformity in five patients (25%). There was accompanying bronchial atresia in two patients and a mild vascular pulsatile mass in one patient (Table 1). There was no microbiologically significant growth in the bronchoalveolar lavage samples, and the cytopathological examination results were normal.
Two patients(10%) who were followed up with a diagnosis of asthma underwent inhaled steroid therapy, and two patients(10%) were diagnosed with wheezing. One patient (5%) was diagnosed with tethered cord syndrome, one patient (5%) was diagnosed with a laryngeal cleft, and one patient (5%) was diagnosed with familial dystonia; 3 patients (15%) presented with CHD. Echocardiography revealed patent ductus arteriosus in one patient (5%), secundum atrial septal defect in one patient (5%), and a small muscular ventricular septal defect in one patient (5%). The patients diagnosed with CHD did not require medical or surgical treatment, and there was no statistically significant difference between the two groups in terms of comorbid diseases (Table 1).
Lobectomy was performed by the surgical department for four symptomatic patients. The first patient was admitted to the pediatric intensive care unit for respiratory distress and pneumonia at the age of three months and for increased aeration in the upper zone of the left lung, causing right upper atelectasis and a mediastinal shift as detected in the patient’s CXRs. The patient was admitted due to their need for invasive positive mechanical ventilation. His left upper lobe was markedly malastic as shown by FB, was diagnosed with CLE and underwent left upper lobectomy. While the second patient was being followed up in the neonatal intensive care unit with 2 L/min of oxygen support through a nasal cannula, he was referred to our clinic as an outpatient at the age of five months with an total hyperinflation of the left lung that did not improve and caused mediastinal shifting. The patient whose FB revealed a fish-mouth deformity in the left lower lobe underwent a left lower lobectomy. The third patient was examined at four months of age due to coughing and wheezing; increased ventilation was observed in the patient’s left lung, causing mediastinal shifting. The FB revealed that the left upper lobe had a fish-mouth shape and that there was a mucosal flap in the lateral part of the lesion. The fourth patient was examined for a lung infection at the age of 15 days and was followed up on an outpatient basis due to increased ventilation in their left lung, causing a mediastinal shift in the patient’s CXRs. The FB revealed a significant fish-mouth deformity in the left upper lobe. Both third and fourth patients with mediastinal shift and frequent lung infections underwent left upper lobectomy within two weeks after the diagnosis.
The pathology evaluation of the lobectomy specimens found enlargement of alveolar structures in four specimens and immature bronchial cartilages in two specimens. Concomitant pulmonary sequestration, congenital airway malformation, and malignancy were not observed.
Although most of the patients who underwent surgery were male, there was no statistically significant difference in sex among the patients who were followed up conservatively. The four patients who underwent lobectomy at diagnosis had a median age of 1.9 months (range, 1 day–4 months), and the most common symptoms were respiratory distress, tachypnea, and retraction. The patients who were followed up conservatively had a median age of 3.16 years (4 days–17 years) at diagnosis, and the most common symptoms were cough. Of the four patients who underwent surgery, three patients (75%) had left upper lobe involvement, and one patient (25%) had left lower lobe involvement. The mediastinal shift was observed in all patients, and secondary atelectasis was observed in two patients in the imaging and in four patients who underwent surgery. Respiratory distress and mediastinal shift were more prominent in the patients who underwent surgery than the patients who were followed up conservatively and diagnosed at an early age (p = 0.001, p = 0.049, p = 0.001, respectively). Although the rate of left upper lobe involvement was higher for the patients who underwent surgery than for those treated conservatively, the difference was not statistically significant (p = 0.067; Table 1).
Of the patients with CLE who were followed up conservatively after diagnosis, four were able to cooperate with the spirometry in the follow-up, one patient had normal spirometry, one had mild obstruction, and two had mild restriction. The patients who underwent surgical lobectomy were aged between 1 and 4 years at their last visit in 2020; therefore, spirometry could not be performed (Table 2).
The median follow-up period was 23.2 months (8 months–7 years). In the check-ups of the four patients who underwent lobectomy, their complaints significantly regressed, their growth and development were normal, and they did not experience pneumonia during their follow-up. There were also no changes except for postoperative changes in their CXRs and increased compensatory aeration on the operated side. Sixteen patients who were conservatively followed up had no complaints in their check-ups and did not have pneumonia or asthma attacks. One patient presented with linear atelectasis, which improved with physiotherapy and postural drainage. None of the patients required oxygen, and none were malnourished. In the last visit, the increase in aeration in the check-up CXRs regressed in all patients and was highly evident in two patients(12.5%; Table 3). None of the patients who were conservatively followed up or who underwent surgery required a repeat CT in their follow-up. There were no deaths in the early or late postoperative period in any of the patient groups.